Article

PKR and the Integrated Stress Response drive immunopathology caused by ADAR1 mutation

2020-12-01

Abstract excerpt

<h4>Summary</h4> Mutations in ADAR , the gene that encodes the ADAR1 RNA deaminase, cause numerous human diseases, including Aicardi-Goutières Syndrome (AGS). ADAR1 is an essential negative regulator of the RNA sensor MDA5, and loss of ADAR1 function triggers inappropriate activation of MDA5 by self-RNAs. However, the mechanisms of MDA5-dependent disease pathogenesis in vivo remain unknown. Here, we introduce a...

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Literature Corpus work: b6638c97-5020-5871-bb53-40e1050d69df
DOI: 10.1101/2020.11.30.405498

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